JORGE ELIAS KALIL FILHO

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Departamento de Clínica Médica, Faculdade de Medicina - Docente
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina
Instituto Central, Hospital das Clínicas, Faculdade de Medicina
LIM/19 - Laboratório de Histocompatibilidade e Imunidade Celular, Hospital das Clínicas, Faculdade de Medicina - Líder

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Revista / Livro: Journal of Clinical Immunology ×

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  • conferenceObject
    Descriptive Review of Clinical Data from 186 Records of Outpatients with IgA Deficiency Accompanied at a Quaternary Hospital in Brazil
    (2014) MENDONCA, L.; MASCARENHAS, F.; COHON, A.; KOKRON, C.; GRECCO, O.; KALIL, J.; BARROS, M.
  • conferenceObject
    FREQUENCY OF LYMPHOMAS IN A COHORT OF PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY
    (2013) SINI, B. C.; KOKRON, C. M.; LEVY, D.; PEREIRA, Juliana; OLIVEIRA, Ana Karolina Barreto; COHON, Andrea; KALIL, Jorge; BYDLOWSKI, S. P.; BARROS, Myrthes Toledo
    Introduction: Common variable immunodeficiency (CVID) is characterized by recurrent infections and in-creased susceptibility to autoimmunity and malignancies. Objective: To evaluate the frequency of lymphoma in a cohort of patients with CVID. Methods: Analysis of data obtained from medical records. Results: We analyzed 144 patients, 80 men and 64 women, aged 15-72 years. Seven (0.5 %) lymphoma cases were diagnosed (1 Hodgkin lymphoma and 6 non-Hodgkin lymphomas), affecting 5 men and 2 women aged 1953 years at lymphoma diagnosis. The time period between the onset of symptoms of CVID and lymphoma diagnosis ranged from 7 to 24 years in 4 patients and in 3 of them the diagnosis of both diseases were nearly overlapped preceding the CVID diagnosis in only 1, 3 and 6 months. Fever, splenomegaly and lymphadenopathy occurred in all patients. Lymphocytosis with inversion of the CD4/CD8 ratio at the expense of increased CD8+ T cells was detected in 2 patients and lymphopenia in one. B lymphocytes were undetectable in 3 patients in whom the diagnosis of lymphoma and CVID were established simultaneously and in one B cells were present at the beginning of CVID but undetectable at diagnosis of lymphoma. Conclusions: Patients with absence of B cells in recently diagnosed CVID or absence of B cells in long term followed CVID with previously detectable B cells demands a screening for the presence of lymphomas.
  • article 15 Citação(ões) na Scopus
    Recessive NLRC4-Autoinflammatory Disease Reveals an Ulcerative Colitis Locus
    (2022) STEINER, Annemarie; REYGAERTS, Thomas; PONTILLO, Alessandra; CECCHERINI, Isabella; MOECKING, Jonas; MOGHADDAS, Fiona; DAVIDSON, Sophia; CAROLI, Francesco; GROSSI, Alice; CASTRO, Fabio Fernandes Morato; KALIL, Jorge; GOHR, Florian N.; I, Florian Schmidt; BARTOK, Eva; ZILLINGER, Thomas; HARTMANN, Gunther; GEYER, Matthias; GATTORNO, Marco; MENDONCA, Leonardo Oliveira; MASTERS, Seth L.
    Purpose NLRC4-associated autoinflammatory disease (NLRC4-AID) is an autosomal dominant condition presenting with a range of clinical manifestations which can include macrophage activation syndrome (MAS) and severe enterocolitis. We now report the first homozygous mutation in NLRC4 (c.478G > A, p.A160T) causing autoinflammatory disease with immune dysregulation and find that heterozygous carriers in the general population are at increased risk of developing ulcerative colitis. Methods Circulating immune cells and inflammatory markers were profiled and historical clinical data interrogated. DNA was extracted and sequenced using standard procedures. Inflammasome activation assays for ASC speck formation, pyroptosis, and IL-1 beta/IL-18 secretion confirmed pathogenicity of the mutation in vitro. Genome-wide association of NLRC4 (A160T) with ulcerative colitis was examined using data from the IBD exomes portal. Results A 60-year-old Brazilian female patient was evaluated for recurrent episodes of systemic inflammation from six months of age. Episodes were characterized by recurrent low-grade fever, chills, oral ulceration, uveitis, arthralgia, and abdominal pain, followed by diarrhea with mucus and variable skin rash. High doses of corticosteroids were somewhat effective in controlling disease and anti-IL-1 beta therapy partially controlled symptoms. While on treatment, serum IL-1 beta and IL-18 levels remained elevated. Genetic investigations identified a homozygous mutation in NLRC4 (A160T), inherited in a recessive fashion. Increased ASC speck formation and IL-1 beta/IL-18 secretion confirmed pathogenicity when NLRC4 (A160T) was analyzed in human cell lines. This allele is significantly enriched in patients with ulcerative colitis: OR 2.546 (95% 1.778-3.644), P = 0.01305. Conclusion NLRC4 (A160T) can either cause recessively inherited autoinflammation and immune dysregulation, or function as a heterozygous risk factor for the development of ulcerative colitis.
  • conferenceObject
    Altered Expression of BAFF-R and TACI in Common Variable Immunodeficiency (CVID) Patients
    (2015) SILVA, Diana Cordeiro de Oliveira; BARSOTTI, Nathalia Silveira; OLIVEIRA, Ana Karolina Barreto de; ZILINSKI, Fernando Ramon; BARROS, Myrthes Toledo; KALIL, Jorge; KOKRON, Cristina Maria
  • conferenceObject
    Complement Activation in a Group of Patients with IGG 4 Related Disease at Hospital Das Clincas - University of Sao Paulo
    (2014) MENDONCA, L.; MASCARENHAS, F.; KOKRON, C.; KALIL, J.; BARROS, M.
  • conferenceObject
    Role of Autoimmunity in Hepatitis C Virus Infection: A Case Report and a Brief Review of Literature
    (2014) LIMA, F. M. S.; MENDONCA, L. O.; KOKRON, C. M.; KALIL, J.; CORDOVA, P. T.; BARROS, M. T.
  • conferenceObject
    CASE REPORT: LEPROSY ASSOCIATED TO COMMON VARIABLE IMMUNODEFICIENCY
    (2013) BARROS, Myrthes Toledo; TRINDADE, M. B.; SINI, B. C.; OLIVEIRA, Ana Karolina Barreto; SANCHEZ, J. A.; KALIL, Jorge; KOKRON, C. M.
    Introduction: Common variable immunodeficiency (CVID) is characterized by recurrent infections and, less often, opportunistic infections. Objective: To report a patient with CVID and leprosy. Case: White male, 25 years, with CVID diagnostic established 2 ½ years before, under IGIV monthly replacement. Two years after the diagnosis of CVID, he reported recent appearance of hypopigmented anesthetic spots in the upper limbs and trunk. Skin biopsy: lymphocytic inflammatory infiltrate with sketches of perivascular and perianexial granulomas attacking nerve fibers in the middle and deep dermis. The bacterial index, the tuberculin skin test and research for HIV were negative. The diagnosis of borderline tuberculoid leprosy was established. The number of TCD4+ cells was lower before (572 cells/mm 3) than after (665 cells/mm 3) skin lesions detection. We found no previous reports in the literature of leprosy associated to CVID. Conclusion: We speculate if in this CVID patient the skin lesions corresponded to inflammatory immune reconstitution syndrome (IRIS) associated to leprosy andif IGIVreplacement could modified the outcome of a previous infection by M. leprae.
  • conferenceObject
    Autoimmunity and Family History of PID as Risk Factors for the Evolution of IgA Deficiency to CVID
    (2013) KOKRON, C. M.; BARROS, M. Toledo; GOMES, J.; GRECCO, O.; OLIVEIRA, A. K. Barreto de; COHON, A.; KALIL, J.
  • article 8 Citação(ões) na Scopus
    Rare Pathogenic Variants in Mitochondrial and Inflammation-Associated Genes May Lead to Inflammatory Cardiomyopathy in Chagas Disease
    (2021) OUARHACHE, Maryem; MARQUET, Sandrine; FRADE, Amanda Farage; FERREIRA, Ariela Mota; IANNI, Barbara; ALMEIDA, Rafael Ribeiro; NUNES, Joao Paulo Silva; FERREIRA, Ludmila Rodrigues Pinto; RIGAUD, Vagner Oliveira-Carvalho; CANDIDO, Darlan; MADY, Charles; ZANIRATTO, Ricardo Costa Fernandes; BUCK, Paula; TORRES, Magali; GALLARDO, Frederic; ANDRIEUX, Pauline; BYDLOWSKY, Sergio; LEVY, Debora; ABEL, Laurent; CARDOSO, Clareci Silva; SANTOS-JUNIOR, Omar Ribeiro; OLIVEIRA, Lea Campos; OLIVEIRA, Claudia Di Lorenzo; NUNES, Maria Do Carmo; COBAT, Aurelie; KALIL, Jorge; RIBEIRO, Antonio Luiz; SABINO, Ester Cerdeira; CUNHA-NETO, Edecio; CHEVILLARD, Christophe
    Cardiomyopathies are an important cause of heart failure and sudden cardiac death. Little is known about the role of rare genetic variants in inflammatory cardiomyopathy. Chronic Chagas disease cardiomyopathy (CCC) is an inflammatory cardiomyopathy prevalent in Latin America, developing in 30% of the 6 million patients chronically infected by the protozoan Trypanosoma cruzi, while 60% remain free of heart disease (asymptomatic (ASY)). The cytokine interferon-gamma and mitochondrial dysfunction are known to play a major pathogenetic role. Chagas disease provides a unique model to probe for genetic variants involved in inflammatory cardiomyopathy. Methods We used whole exome sequencing to study nuclear families containing multiple cases of Chagas disease. We searched for rare pathogenic variants shared by all family members with CCC but absent in infected ASY siblings and in unrelated ASY. Results We identified heterozygous, pathogenic variants linked to CCC in all tested families on 22 distinct genes, from which 20 were mitochondrial or inflammation-related - most of the latter involved in proinflammatory cytokine production. Significantly, incubation with IFN-gamma on a human cardiomyocyte line treated with an inhibitor of dihydroorotate dehydrogenase brequinar (enzyme showing a loss-of-function variant in one family) markedly reduced mitochondrial membrane potential (Delta psi M), indicating mitochondrial dysfunction. Conclusion Mitochondrial dysfunction and inflammation may be genetically determined in CCC, driven by rare genetic variants. We hypothesize that CCC-linked genetic variants increase mitochondrial susceptibility to IFN-gamma-induced damage in the myocardium, leading to the cardiomyopathy phenotype in Chagas disease. This mechanism may also be operative in other inflammatory cardiomyopathies.
  • conferenceObject
    Evaluation of Clinical and Laboratory Response to Influenza and H1N1 Vaccines in a Group of Brazilian CVID Patients
    (2012) PEDRESCHI, Maira; OLIVEIRA, Ana Karolina Barreto; BARROS, Myrthes Toledo; COHON, Andrea; CELESTINO, A. T.; VILAS-BOAS, L. S.; FELIX, A. C.; PANNUTI, C. S.; KALIL, Jorge; KOKRON, Cristina M.
    CVID pts lack vaccine-specific ab production. We studied the clinical / laboratorial responses to influenza and H1N1 immunization in 22 CVID pts. Clinical evaluation was done through a score (infections, hospitalizations, antibiotic use). The score was applied during the previous and following year post immunization (PI). Blood was drawn before and 1/3/6/12 m PI. A significant reduction was observed in upper respiratory infections and sinusitis in the year PI (p<0.001), and 6 pts seroconverted. Statistical analysis showed no difference among ab levels before and PI. Among the 9 pts who presented H1N1 ab production, 4 reduced the clinical score. Of note, 50% of the 22 pts presented reduction of clinical scores. Then, although ab production to H1N1 wasn’t statistically significant, we observed a reduction in the number of infections in the year PI. This observation may be due to eventual presence of influenza’sabs but reinforces potential benefits of vaccination in CVID pts.